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At the time the article was created Frank Gaillard had no recorded disclosures.View Frank Gaillard's current disclosures
At the time the article was last revised Sonam Vadera had no recorded disclosures.View Sonam Vadera's current disclosures
Gardner syndrome is one of the polyposis syndromes. It is characterized by:
- familial adenopolyposis
- multiple osteomas: especially of the mandible, skull, and long bones
- epidermal cysts
- desmoid tumors of mesentery and anterior abdominal wall
Other abnormalities include:
- supernumerary teeth, odontomas and dentigerous cysts 4,5
- duodenal tumors / ampullary carcinoma 2,3
- papillary thyroid carcinoma
There is an autosomal dominant inheritance in the FAP gene (chromosome 5q) in a majority of patients but with 20% of cases resulting from new mutations. Extracolonic features often precede the diagnosis of colonic polyps.
History and etymology
First described in 1953 by Gardner and Richards 3.